Atretic aortic valvular cusp causing severe aortic regurgitation

Atretic aortic valvular cusp causing severe aortic regurgitation Case report  We report a case of a 19 year-old man who presented with murmur for investigation. He was previously followed up by paediatrician for aortic regurgitation (AR) but later defaulted clinics. He was otherwise asymptomatic and physically active. On clinical examination, he had a collapsing pulse. There was a loud 4/6 to-and-fro murmur best heard over the right upper sternal edge. The apex beat was displaced laterally to sixth intercostal space along mid-clavicular line. Echocardiography showed dilated left ventricle (LV, internal diastolic diameter 70 mm), and bidirectional eccentric AR. The left ventricular ejection fraction (LVEF) was 50%, AR vena contracta was 8.8 mm, and pressure half time measured 350 m/s; there is holo-diastolic flow reversal in the descending thoracic and abdominal aorta. Transoesophageal echocardiogram confirmed an atretic right coronary cusp (RCC) and an enlarged asymmetric non-coronary cusp (NCC) which allowed bidirectional flow of cardiac output and AR though the orifice (Figure 1A–D, Supplementary data online, Videos S1–S3). View largeDownload slide View largeDownload slide Computed tomography (CT) aortogram showed morphologically trileaflet aortic valve with atretic RCC. Normal origins of the right and left coronary arteries were observed. The aortic annulus, sinus and sinotubular junction measured 33 mm, 33 mm, and 30 mm, respectively. The proximal ascending aorta (45 mm) and proximal arch (35 mm) were dilated (Figure 1E). The final diagnosis is congenital AR caused by rudimentary RCC. Surgery  He subsequently underwent a corrective aortic valve replacement with size 22 metallic ATS Medical valve. RCC was confirmed to be atretic. Post-surgery echocardiography showed LVEF of 35%, though he had no failure symptoms. Supplementary data are available at European Heart Journal - Cardiovascular Imaging online. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2018. For permissions, please email: journals.permissions@oup.com. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices) http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png European Heart Journal – Cardiovascular Imaging Oxford University Press

Atretic aortic valvular cusp causing severe aortic regurgitation

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Publisher
Oxford University Press
Copyright
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2018. For permissions, please email: journals.permissions@oup.com.
ISSN
2047-2404
D.O.I.
10.1093/ehjci/jey074
Publisher site
See Article on Publisher Site

Abstract

Case report  We report a case of a 19 year-old man who presented with murmur for investigation. He was previously followed up by paediatrician for aortic regurgitation (AR) but later defaulted clinics. He was otherwise asymptomatic and physically active. On clinical examination, he had a collapsing pulse. There was a loud 4/6 to-and-fro murmur best heard over the right upper sternal edge. The apex beat was displaced laterally to sixth intercostal space along mid-clavicular line. Echocardiography showed dilated left ventricle (LV, internal diastolic diameter 70 mm), and bidirectional eccentric AR. The left ventricular ejection fraction (LVEF) was 50%, AR vena contracta was 8.8 mm, and pressure half time measured 350 m/s; there is holo-diastolic flow reversal in the descending thoracic and abdominal aorta. Transoesophageal echocardiogram confirmed an atretic right coronary cusp (RCC) and an enlarged asymmetric non-coronary cusp (NCC) which allowed bidirectional flow of cardiac output and AR though the orifice (Figure 1A–D, Supplementary data online, Videos S1–S3). View largeDownload slide View largeDownload slide Computed tomography (CT) aortogram showed morphologically trileaflet aortic valve with atretic RCC. Normal origins of the right and left coronary arteries were observed. The aortic annulus, sinus and sinotubular junction measured 33 mm, 33 mm, and 30 mm, respectively. The proximal ascending aorta (45 mm) and proximal arch (35 mm) were dilated (Figure 1E). The final diagnosis is congenital AR caused by rudimentary RCC. Surgery  He subsequently underwent a corrective aortic valve replacement with size 22 metallic ATS Medical valve. RCC was confirmed to be atretic. Post-surgery echocardiography showed LVEF of 35%, though he had no failure symptoms. Supplementary data are available at European Heart Journal - Cardiovascular Imaging online. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2018. For permissions, please email: journals.permissions@oup.com. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices)

Journal

European Heart Journal – Cardiovascular ImagingOxford University Press

Published: Sep 1, 2018

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